Case Reports of a New Syndrome Associating Gingival Fibromatosis and Dental Abnormalities in a Consanguineous Family

Martelli‐Júnior, Hercílio; Bonan, Paulo Rogério Ferreti; Santos, Luís; Santos, Silvânia Paiva dos; Cavalcanti, Marcelo Gonçalves; Coletta, Ricardo D.

doi:10.1902/jop.2008.070520

Cited by 57 publications

(72 citation statements)

References 39 publications

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“…This is an autossomal, recessive and rare disease, characterized by mild gingival hyperplasia and dental abnormalities, including generalized hypoplastic AI, intrapulpar calcifications and delayed dental eruption. A similar case was described by Martelli-Junior et al 17 where four patients of the same family had the same abnormality and one patient had intellectual deficit. AI diagnosis is slightly complex, because it presents a set of problems, such as rampant caries, impairment of vertical occlusion dimension, eruption abnormalities, dental sensitivity, in addition to psychosocial problems 18 .…”

Section: Discussionsupporting

confidence: 74%

Intracannal pain remission in child with amelogenesis imperfecta. Case report

Nascimento

Barros²,

Soares³

et al. 2015

Revista Dor

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Section: Discussionsupporting

confidence: 74%

Intracannal pain remission in child with amelogenesis imperfecta. Case report

Nascimento

Barros²,

Soares³

et al. 2015

Revista Dor

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“…HGF can vary from focal sites of gingival enlargement to generalised involvement, with the degree of overgrowth varying from slight to severe. 12 Unlike drug-induced gingival overgrowth, HGF is not influenced by plaque, and the incidence and severity of the disease appears to depend on the penetrance of the mutated gene. 8 The patients reported here exhibited a generalised and severe gingival overgrowth.…”

Section: Discussionmentioning

confidence: 99%

“…It is evident from the previous reported cases that the condition usually begins at the time of eruption of the permanent dentition, but it can develop with the eruption of the deciduous dentition and is even more rarely seen at birth. 12 In a study involving 17 family members with gingival fibromatosis, Fletcher reported that the most extensive enlargement appeared to occur either during loss of the deciduous teeth or in early stages of eruption of the permanent dentition. 4 He noted that the enlargement seemed to progress rapidly during "active" eruption and decrease with the end of this stage.…”

Section: Discussionmentioning

confidence: 99%

Extensive Gingival Enlargement in Siblings : A Case Report = ضخامة اللثة الشاملة عند الأشقاء : تقرير حال

Pushpanshu¹,

Kaushik²,

Sathawane³

et al. 2012

SQUMJ

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Gingival hyperplasia is an unusual condition which interferes with speech, mastication, occlusion, lip continence, and facial appearance of the affected individual, causing aesthetic, functional, psychological, and masticatory disturbances. Drug-induced gingival enlargements are the commonest form. The inherited condition in which the gingival tissue spontaneously and progressively enlarges is identified as hereditary gingival fibromatosis (HGF).1 It is a rare condition, affecting only one in 750,000 people, with males and females being equally affected.2 HGF is characterised by a slowly progressive, non-haemmorhagic fibrous enlargement of gingival tissue, occurring peripheral to the alveolar bone, which does not involve the periodontal ligament. The hyperplastic gingival tissue usually presents with a normal colour and has a firm consistency with abundant stippling. 3,4 Furthermore, HGF usually develops as an isolated disorder but can be one feature of a syndrome or a chromosomal abnormality. Accordingly, it has been divided into two forms: non-syndromic and syndromic. 5The gingival enlargement usually begins with the eruption of the permanent or deciduous dentition. 1The growth may worsen throughout adolescence, suggesting an influence of growth hormones. 6 The clinical presentation of HGF is highly variable, both in the number of teeth involved and degree of Sultan Qaboos University Med J, November 2012, Vol. 12, Iss. 4, pp. 517-521, Epub. 20 th Nov 12 The condition may be found in an autosomal dominant or autosomal recessive mode of inheritance, the former being more common. It usually develops as an isolated disorder but can be one feature of a multisystem syndrome. Accordingly, HGF has been divided into two forms: non-syndromic and syndromic. The gingival enlargement can be localised or generalised, but usually involves both arches. The authors describe a case of non-syndromic generalised severe HGF, involving the maxillary and mandibular arches in two brothers. This report focuses on the diagnosis, treatment, and control of the disease. The pattern of inheritance and histopathologic characteristics are also emphasised.

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“…8 Clinical examination of the 70 family members revealed 1 member affected by isolated GF, 3 affected by GF and DA, and 1 member with GF and DA in association with mental retardation (MR). Six members presented with isolated MR. All affected members came from consanguineous marriage, except for the member with isolated GF.…”

Section: Subjectsmentioning

confidence: 99%

“…7 Recently, we described a new association of GF and dental abnormalities (DA), including generalized thin hypoplastic amelogenesis imperfecta (AI). 8 The clinical expression of GF is highly heterogeneous and the gingival enlargement results in both aesthetic and functional problems for affected individuals. 9 There are few studies reporting imaging alterations associated with FG as this is a condition that does not directly affect hard tissue.…”

Section: Introductionmentioning

confidence: 99%

Imaging evalution of the gingival fibromatosis and dental abnormalities syndrome

Neto

Santos²,

Coletta³

et al. 2011

Dentomaxillofacial Radiology

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Objective: The purpose of this study was to evaluate the dentomaxillofacial imaging features of one family affected by the gingival fibromatosis (GF) and dental abnormalities (DA) syndrome. Methods: Conventional radiographs (periapical and panoramic) and cone beam CT (CBCT) were performed in nine members of this family: four were affected by the syndrome and five were not. Results: The four affected members demonstrated mild generalized GF in association with DA, including hypoplastic amelogenesis imperfecta, intrapulpal calcifications, delay on tooth eruption and pericoronal radiolucencies in unerupted teeth. None of these oral changes were identified in the five unaffected members. All nine members presented alterations in the paranasal sinuses and mucosal thickening of the maxillary sinus was the most common finding. Conclusion: Family members not affected by the syndrome showed similar alterations in the paranasal sinuses and CBCT was useful to characterize the dentomaxillofacial features of this new syndrome associating GF and DA.

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Case Reports of a New Syndrome Associating Gingival Fibromatosis and Dental Abnormalities in a Consanguineous Family

Abstract: To the best of our knowledge, these cases represent a new syndrome within the spectrum of those including GF.

Cited by 57 publications

References 39 publications

Intracannal pain remission in child with amelogenesis imperfecta. Case report

Intracannal pain remission in child with amelogenesis imperfecta. Case report

Extensive Gingival Enlargement in Siblings : A Case Report = ضخامة اللثة الشاملة عند الأشقاء : تقرير حال

Imaging evalution of the gingival fibromatosis and dental abnormalities syndrome

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